Venous Malformations: A Real-Life Use of DOAC for Pain Reduction and Thrombosis Prevention

Venous malformations (VMs) are frequent congenital lesions, resulting from venous dysmorphogenesis, and are mostly sporadic. The major symptom reported by patients is pain, notably related to thrombosis. Coagulation disorders are also encountered, such as the localized intravascular coagulopathy (LIC). In case of severe LIC, low molecular weight heparin is the first-line treatment, but some studies suggest that direct oral anticoagulants (DOACs) have good efficacy and safety. Our objective was to evaluate the efficacy and safety of DOAC treatment on pain in VM in a pragmatic cohort. We included 117 patients with a VM; the mean age at diagnosis was 16 years (SD 4.5). The median follow-up time was 5.6 years (3.1–9.4). Anticoagulant treatment was introduced in 57 patients (48.7%), a DOAC in 55. Among patients reporting pain, there were significantly more localizations in the lower limbs (76.4% vs 42.2%, P < .001), muscle involvement (68.1% vs 46.7%, P = .036), and previous local thrombosis (66.7% vs 20%, P < .001). Among patients who received anticoagulation, 53 patients reported pain before treatment, moderate in 16 patients (30%) and severe in 37 patients (70%). After the introduction of DOACs, 22 patients reported pain (41%), severe in 5 patients (11%). One patient (1.9%) presented a local venous thrombosis, and 1 patient (1.9%) presented a deep vein thrombosis and a pulmonary embolism. No major bleedings were reported. Our study suggests that DOAC treatment is often prescribed in VMs with a reduction in pain and good tolerance. Further studies are needed to better evaluate the best treatment regimen in these patients.